BackgroundBetween 2002-2011, the incidence of severe primary postpartum hemorrhage (PPH) in Dutch women with Von Willebrand disease (VWD) and hemophilia carriers (HCs) was 8% vs. 4.5% in the general population. AimTo determine the contemporary incidence of severe primary PPH in women with VWD and HCs. MethodsAll women with VWD or HCs who delivered between 2012-2017 were selected from all six Dutch Hemophilia Treatment Centers. Patient/disease characteristics, peripartum hematologic/obstetric management and outcomes were retrospectively collected. Incidence of severe primary (≥1000 mL blood loss ≤24hrs) and primary (≥500 mL ≤24hrs) PPH was compared with the 1) previous cohort and 2) general Dutch population, and between 3) women with VWD and HCs with third trimester coagulation activity levels <50 IU/dL vs. ≥50 IU/dL and 4) women treated with vs. without peripartum hemostatic prophylaxis. Results348 deliveries (151 VWD, 167 hemophilia A and 30 hemophilia B carriers) were included. The severe primary PPH incidence was 10% (36/348) and remained stable over time, whereas this incidence has increased in the general population (to 8%) leading to a similar risk (p=0.17). Severe primary PPH risk was comparable between women with coagulation activity levels <50 vs. ≥50 IU/dL (11% (7/66) vs. 10% (29/279), Odds Ratio (OR) 1.02; 95% Confidence Interval (CI): 0.43-2.44) and comparable between those with vs. without prophylaxis (12%, 11/91 vs. 10%, 25/254, OR: 1.26; 95% CI 0.59-2.68). ConclusionsSevere primary PPH in women with VWD and HCs remained stable and is comparable with the increasing prevalence in the general population. More research is needed to find the optimal pregnancy management strategy for safe delivery in VWD and HC.